Ehler’s-Danlos Syndrome; excessive dynamic airway collapse; hypertrophy of the trachealis and bronchialis muscles

Spasm of muscle [M62.838]Subglottic stenosis [J38.6]
Chronic respiratory failure, unspecified whether with hypoxia or hypercapnia (HCC) [J96.10]

Suspension MicrolaryngoscopyBronchoscopy with Botox injection of trachealis/bronchialis muscles.
Bronchoscopy with CO2 laser scarification of Posterior Bronchial/Tracheal Wall

Anesthesia: General

Estimated Blood Loss: Minimal

Drain: NA

Total IV Fluids: as per anesthesia

Specimens: * No specimens in log *

Implants: * No implants in log *

Complications: 3 mm anterior tongue laceration sustained during exposure with Lindholm scope

Findings:
1) Moderate difficulty with Lindholm scope, placed in suspension
2) Stable tracheal stenosis, minimally obstructive without intervention necessary today
3) Improvement in bulk of trachealis and bronchialis from prior botox and laser procedures, but overall still with some hypertrophy
4) Around 20-25 U botox injected to right and left mainstem bronchus bronchialis muscle each using sclerotherapy needle. Another 50-60 U botox injected to trachealis in multiple locations.
5) Flexible CO2 laser with flexible bronchoscopy used to scar distal posterior tracheal wall and right mainstem posterior bronchial wall (left side left untouched as it appeared less hypertrophied and sufficiently open)

Disposition: awakened from anesthesia, extubated and taken to the recovery room in a stable condition, having suffered no apparent untoward event.

Condition: doing well without problems

Technique: clean, contaminated

Procedure Details: Patient was brought into operating room and turned over to Anesthesia. After timeout was performed, patient was induced under general anesthesia and bag masked with no difficulty. Patient was then turned over to the ENT team, and we performed bag mask ventilation with ease. Mouth guard was put over patient’s maxillary teeth for protection, and the Lindholm scope was then used to expose the larynx with moderate difficulty. The patient was placed in suspension with the Lewis arm. The zero degree hopkins rod was used to visualize the larynx, however, the angle of suspension did not allow for visualization of the trachea. Jet ventilation was initiated through the scope port, but patient’s saturations were not sustained, as such, the patient was intubated with a 5-0 ET tube with no difficulty through the scope. After ventilating sufficiently, tube was taken out, and jet ventilation was reinitiated by extending a catheter below the glottis. The flexible bronchoscope was used to examine the airway. There was persistent stable tracheal stenosis that was only minimally obstructive. As such, no intervention was deemed necessary. The trachealis and bilateral mainstem bronchialis muscles were noted to still be hypertrophied as before, though seemingly less so (particularly the left mainstem bronchus). The previous laser marks were no longer visible in the posterior wall, but there did appear to be a drop off where the laser marks had ended previously (c/w a reduction in bulk of the muscle where laser had been used). After examination, jet ventilation was held, and the ET tube was re-inserted, and the patient was ventilated by Anesthesia. The botox was then prepared to a concentration of 50 U/mL. This was placed in a 5cc syringe attached to a sclerotherapy needle, which was threaded into the flexible bronchoscope. When Anesthesia had deemed patient ready for jet ventilation again, the ET tube was removed, jet ventilation was initiated, and the flexible bronchoscope was used to direct the needle towards the trachealis and bronchialis muscle. A total of about 20-25U was injected into each mainstem bronchus bronchialis muscle, and about 50-60U into the main trachealis muscle in multiple locations. Methylene blue was injected into the sclerotherapy catheter after the botox syringe ran out in order to determine when the botox was all used up. After this was completed, the patient was re-intubated and ventilated by Anesthesia.
Then, patient’s face was draped with wet towels, and the CO2 laser was prepared. Patient was extubated, and jet ventilation initiated again with transglottic catheter. All personnel in the OR at this time donned the appropriate eye protective wear. The laser flexible catheter was threaded through the flexible bronchoscope, which was then advanced into the trachea, and two longitudinal furrows were made over the right mainstem posterior bronchial wall extending up into the distal posterior tracheal wall. the jet ventilator was used at a laser safe mode while using the CO2 laser. The bronchoscope was withdrawn, the vocal cords were sprayed with 4% lidocaine, and the patient was re-intubated. Long grabbing forceps were used to hold the ET tube in place while the Lindholm scope was removed from the patient’s mouth, and patient was handed over to Anesthesia. The case was deemed to be finished at this point. All counts were correct at the end of the case. Patient was extubated and awakened from anesthesia without complication.

Many thanks, Jamie